Ring chromosome 11. A case report and review of the literature

A female infant with severe growth-weight retardation and with a ring chromosome 11, associated with trisomy X in 15% of metaphases, has been reported. A literature review of cases of r(11) shows that the clinical features of these patients, although showing different frequencies, are similar to those of the del(11q) syndrome. It has been suggested that the variability of the mental retardation in r(11) patients is attributable to the unstability of the ring and to the different break points in these two chromosomal rearrangements. The origin of the r(11) was also addressed by studying fragile sites of the parents at 11p15 and 11q25.     

Treatment of promyelocytic leukemia during pregnancy. A case report and review of the literature

In spite of the fact that acute promyelocytic leukemia during pregnancy is rare and that there is little precedent in the literature for treatment with combined chemotherapeutic agents, the rate of success with current chemotherapeutic regimens is very encouraging. Judging from previous reports and our own experience, it is possible to give combination chemotherapy to pregnant women with AML/APL with the result that mother and infant survive, whereby the incidence of complication is within an acceptable range. No comprehensive studies on life-time teratogenic or carcinogenic effects are available at present.     

Nasopharyngeal mucoepidermoid carcinoma: A case report and review of literature

Background

Salivary gland-type tumors originating in the nasopharynx are rare, and only a few articles about mucoepidermoid carcinomas (MEC) in this location have been reported. We describe one case of nasopharyngeal MEC and, based on a review of the literature, discuss different therapeutic approaches that can be taken regarding the result of histological findings, radiological tests and extent of disease.

Case presentation

A 47-year-old woman diagnosed with mucoepidermoid carcinoma of nasopharynx, T1 N3 M0 (stage IV-B) was treated in 2007 with a combination of radiotherapy and chemotherapy to a maximum dose of 70 Gy and concomitant Cisplatin during the radiation. One year later, with the head and neck disease under control, mediastinal nodes relapse appeared which were treated with exclusive radiotherapy to a maximum dose of 65 Gy. One year after the first relapse, a second relapse was detected in the right lung, next to the previously treated mediastinal regions, and the patient initiated a treatment with exclusive chemotherapy based on TPF scheme.

Conclusion

For limited or resectable MEC, combined surgery with radiotherapy, or radiochemotherapy, should be considered the main treatment policy. On the other hand, in poorly differentiated, unresectable tumors or nasopharyngeal MEC, radiochemotherapy could be currently the main treatment approach.     

Rhinosporidiosis in Egypt: A case report and review of literature

Rhinosporidiosis is an infection caused by Rhinosporidium seeberi that frequently presents as a polypoidal nasal lesions. Here, we report the first indigenous case of tumoral rhinosporidiosis in Egypt. In this case, a 25-year-old male patient from a rural background of Assuit City presented with epistaxis and a nasal polyp. The patient had not traveled abroad. The diagnosis was established on the morphological basis by the identification of 5- to 10-m endospores and 50- to 1000-m sporangia. The clinicopathological and immunologic features were discussed and the literature was reviewed. To the best of our knowledge this is the first case of this disease to be reported in Egypt in the human literature.     

Biopsy proven metastatic meningioma: A case report and review of the literature

Meningiomas are the most common type of benign tumor found in the brain and are typically benign, slow-growing lesions. The current standard of care consists of surgical resection and subsequent postoperative radiotherapy to prevent local recurrence. Because of their indolent nature, meningiomas are rarely found to spread extracranially and develop distant metastases. We present the clinical, imaging, and pathologic features of a patient who had meningioma with multiple local recurrences, who was incidentally found to have metastatic disease in the lungs. In addition, we discuss details of this case in the context of the previously reported literature.     

Mediastinal pancreatic pseudocyst--a case report and review of the literature

Pancreatic pseudocyst, a common complication of acute or chronic pancreatitis, in rare instances may also extend to the mediastinum. A case of 67-year-old woman presenting with a triad of chest pain, dysphagia, and dyspnea is presented. The patient had an episode of acute alcoholic pancreatitis 1 year before presentation. Chest radiography on admission showed a retrocardiac opacity. Two-dimensional echocardiography revealed an echolucent mass compressing the left atrium. A subsequent upper gastrointestinal series for her dysphagia showed extrinsic compression of the distal esophagus. Finally a definitive diagnosis was made with computed tomography (scan), which revealed a 19 x 12 cm pseudocyst extending from the body of pancreas into the thorax and compressing the esophagus and the cardiac chambers. A mediastinal pseudocyst can cause symptoms due to compression or invasion of surrounding structures. The fluid collection may enlarge slowly and hence the symptoms can be delayed as in our patient. The pseudocyst was successfully treated using endoscopic ultrasound-guided transesophageal drainage. Approximately 50 cases of mediastinal extension of the pancreatic pseudocyst in the world literature are reported. At this time, this is only the second time that successful drainage of a mediastinal pseudocyst using a transesophageal approach under endoscopic ultrasound guidance has been reported. The literature was reviewed for clinical presentation, complications, and available treatment options for mediastinal pancreatic pseudocysts.     

Eccrine porocarcinoma of the lower extremity: A case report and review of literature

Hepatoid adenocarcinoma is a rare variant of extrahepatic adenocarcinoma which behaves like hepatocellular carcinoma in morphology and functionality. We present a rare case of hepatoid adenocarcinoma of the gallbladder which invades deeply the liver bed, in a 59-year-old woman. Histologically, most of the mass in the gallbladder was composed of cells with eosinophilic cytoplasm arranged in a trabecular pattern, which resembled hepatocellular carcinoma. The main differential diagnosis was hepatocellular carcinoma with invasion into the gallbladder. The gallbladder origin of the hepatoid adenocarcinoma was verified by the presence of foci of conventional adenocarcinoma, the recognition of high-grade dysplasia in the adjacent epithelium and the absence of cirrhosis.     

Cutaneous ciliated cyst: case report and literature review

An 18-year-old girl had a cyst excised from the left buttock in 1988. The cyst was diagnosed to be a cutaneous ciliated cyst, since histologic examination using special stains demonstrated its lining to be similar to fallopian tube epithelium. Only 20 such cases have been published to date, and this report is probably the first case in the plastic surgical literature, to the best of our knowledge.     

Spontaneous remission of acute myeloid leukemia. A report of a case and brief review of the literature

A patient with acute myelomonocytic leukemia who experienced a spontaneous remission, is reported. He had precedent and concurrent bacterial infections as most of these cases described. Low peripheral WBC and myeloblasts, Auer-rod positive blasts, bone marrow eosinophilia with atypical eosinophils, and a partial deletion of chromosome 16 were favorable prognostic parameters. A brief review of the literature and possible explanations for the regulation of granulopoiesis are presented.     

Retroperitoneal primitive neuroectodermal tumour (PNET). A case report and review of the literature

PurposeWe report a clinical case and present a brief review of the literature of peripheral primitive neuroectodermal tumour (PNET) as a rare disease. We discuss the difficult clinical and pathological diagnosis and the multidisciplinary approach to treatment of PNET. We debate radiosensitivity of extracranial recurrent retroperitoneal PNET.Methods and materialsExternal beam radiation therapy was applied for a non-resectable local recurrence of retroperitoneal PNET in a 74-year-old woman. There were no distant metastases and our patient has refused chemotherapy.ResultsLocal tumour control (LTC) was achieved after administration of a total dose of 60 Gy in 30 fractions by external beam 60 Cobalt radiotherapy.ConclusionsPNET is an aggressive malignant tumour infiltrating lymphatics and metastasizing haematogenously. It requires a multimodality treatment. Late local recurrence of extracranial retroperitoneal PNET has shown high radiosensitivity, so local tumour irradiation could be a radical treatment even in non-resectable cases.     

Hemichorea caused by nonketotic hyperosmolar state: A case report and review of the literature

Our report involves a case of hemichorea caused by the nonketotic hyperosmolar state. We have analyzed the clinical data and relevant features of a patient who presented herself to the Affiliated Hospital of Xuzhou Medical University. The patient had unilateral involuntary movements for 1?month. We discovered that her blood glucose levels were very high. The patient underwent computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA), indicating right basal ganglia lesion. Control of the patient’s blood glucose plus supportive treatment resulted in a significant improvement of her clinical state.     

Vulvar syringoma,report of a case and review of the literature

Syringomas are common intraepidermal sweat gland tumors most often found in women around the time of adolescence. Frequent sites of involvement include the lower eyelids and malar areas, however vulvar involvement is relatively rare. These lesions often present as small, multiple, skin-colored-to-yellowish papules and are often associated with increased vulvar discomfort and itching. We present a case of a 29-year old female who presented to her gynecologist complaining of vulvar itching and burning. A small condylomatous-type wart observed on her vulva was biopsied and found to be a syringoma. Because of their clinical presentation and associated symptoms, vulvar syringomas should be considered in the differential diagnosis of any multicentric papular lesion of the vulva, vulvar pain syndrome, and pruritis vulvae.     

Ganglions of the temporomandibular joint: case report and review of literature

We present the third reported case of a ganglion cyst arising from the temporomandibular joint. Like the previous two cases, this lesion occurred in a woman over the age of 40. Unlike the lesions in previous reports, the cyst in our patient was not associated with pain or tenderness, making the diagnosis of parotid tumor more likely. Nevertheless, the case serves to illustrate the need for including ganglion in the differential diagnosis of well-encapsulated preauricular masses in the region of the temporomandibular joint.     

Ectopic ACTH syndrome due to pheochromocytoma: case report and review of the literature.

A 51-year-old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH. This diagnosis was based upon her paroxysmal hypertension, hyperpigmentation, and hypokalemia. Elevated levels of serum and urine corticosteroids, plasma ACTH, urinary VMA, and catecholamines fell after a right adrenal pheochromocytoma was removed. Subsequently this tumor was found to have a high content of ACTH. Review of the literature indicates a mortality rate of 57% for this syndrome. Proper preoperative recognition and management can result in total cure.